C16orf57 Antikörper (AbBy Fluor® 647)

Produktnummer ABIN1403760

Produktdetails anti-C16orf57 Antikörper (AbBy Fluor® 647)

Target: C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser C16orf57 Antikörper ist konjugiert mit AbBy Fluor® 647

Applikation: Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Kreuzreaktivität: Human, Maus, Ratte

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human C16orf57

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

Vorsichtsmaßnahmen: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu C16orf57

Target: C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))

Andere Bezeichnung: C16orf57 (USB1 Produkte)

Synonyme: C16orf57 antikoerper, HVSL1 antikoerper, Mpn1 antikoerper, PN antikoerper, hUsb1 antikoerper, C18H16orf57 antikoerper, AA960436 antikoerper, RGD1305215 antikoerper, c16orf57 antikoerper, C2H16orf57 antikoerper, zgc:91896 antikoerper, U6 snRNA biogenesis phosphodiesterase 1 antikoerper, U6 snRNA biogenesis 1 antikoerper, U6 snRNA biogenesis phosphodiesterase 1 L homeolog antikoerper, USB1 antikoerper, Usb1 antikoerper, usb1.L antikoerper, usb1 antikoerper

Hintergrund:

Synonyms: Chromosome 16 open reading frame 57, CP057_HUMAN, FLJ13154, UPF0406 protein C16orf57.

Background: Involvement in disease,Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.

Gen-ID: 79650